FcRγ-dependent immune activation initiates astrogliosis during the asymptomatic phase of Sandhoff disease model mice

Sandhoff disease (SD) is caused by the loss of β-hexosaminidase (Hex) enzymatic activity in lysosomes resulting from Hexb mutations. In SD patients, the Hex substrate GM2 ganglioside accumulates abnormally in neuronal cells, resulting in neuronal loss, microglial activation, and astrogliosis. Hexb(−...

Descripción completa

Detalles Bibliográficos
Autores principales: Ogawa, Yasuhiro, Sano, Takafumi, Irisa, Masahiro, Kodama, Takashi, Saito, Takahiro, Furusawa, Eiri, Kaizu, Katsutoshi, Yanagi, Yusuke, Tsukimura, Takahiro, Togawa, Tadayasu, Yamanaka, Shoji, Itoh, Kohji, Sakuraba, Hitoshi, Oishi, Kazuhiko
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group 2017
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5234013/
https://www.ncbi.nlm.nih.gov/pubmed/28084424
http://dx.doi.org/10.1038/srep40518

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5234013/
https://www.ncbi.nlm.nih.gov/pubmed/28084424
http://dx.doi.org/10.1038/srep40518

Ejemplares similares